Takayasu's arteritis (TA) is a vasculitis that is found more commonly in women than in men, with 80% to 90% of people with the disease being women. People who get the disease are usually between the ages of 10 and 40 years when the disease starts. The disease affects people around the world, but is most common in Asians, especially in Japan.
TA primarily affects the aorta, the major vessel that carries blood that supplies oxygen to the body. Inflammation from the disease may be localized to one small part of the aorta, or may involve the entire length of the vessel. As the disease progresses, other major blood vessels may also become affected with inflammation. The abdominal aorta, the part of the aorta that runs through the middle of the body and supplies blood to the stomach area, and lung arteries are involved in about one half (50%) of patients. The inflammatory process causes the walls of the aorta to become thick, which makes it difficult for blood to flow easily. This can cause many different symptoms because it depends on where in the body the blood flow is affected and which organs may not get all the blood that they need to work right.
The cause of or reason that TA develops is not understood, although genetics have been associated with increased frequency in several studies.
Symptoms: Symptoms that are common when TA first develops include lack of energy, weight loss, weakness and low-grade fever. Symptoms that affect the blood vessels take time to develop. As the disease progresses the arms and legs may become cool, and pain may develop with use. Other symptoms include joint pain, muscle weakness and pain, and skin lesions. Symptoms of the disease can also include chest pain, shortness of breath and coughing up blood if the lungs become affected. Shortness of breath can develop if the heart is affected. Anemia (low blood count) is present in most patients and results in a general feeling of weakness and tiredness.
Involvement of the major blood vessel that carries blood to the head and brain (the carotid artery) causes decreased blood flow that can lead to dizziness, fainting, headaches, seizures and difficulties thinking and remembering. Difficulty with seeing may also develop. Stomach pain, diarrhea, and stomach bleeding can result from inflammation in the stomach or intestines. Inflammation of the vessels that supply the heart (coronary arteries) can cause chest pain and puts patients at higher risk of having a heart attack (myocardial infarction).
Diagnosis: In order for doctors to see what is happening to the aorta and other blood vessels, several different types ‘pictures' may be taken. Radiography (x-ray) of the chest may be used to look for changes in the aorta. Other types of pictures require that you drink or be given a shot of
something called ‘contrast' material. This material shows up on the picture and helps to show different parts of the inside of your body. These tests are called contrast angiography or arteriography, and they are especially useful to help determine the location and appearance of vessels affected by the disease.
Computed tomographic (CT) or magnetic resonance (MR) imaging scans of the chest, abdomen, head and neck, or other areas may also be used, particularly so that doctors can evaluate large arteries. Positron emission tomography (PET scanning) can also be used to take pictures of the aorta and other large vessels. Several of these types of tests and pictures may be needed in order to diagnose and manage TA.
The main drug used for treatment of TA is corticosteroids, also known as glucocorticoids. Treatment with this medicine usually stops this disease from getting worse by reducing the inflammation associated with the disease. However, use of this medicine, especially for long periods of time, can cause many adverse reactions or side-effects.
Approximately one-half of all patients with TA have disease that is ongoing and does not get completely better with the use of corticosteroids. When this is the case, other drugs such as methotrexate, cyclophosphamide, and azathioprine may reduce inflammation and be helpful in controlling symptoms of the disease. A new drug that may be helpful for patients with TA who do not get better with the treatments listed above is called an anti-tumor necrosis factor alpha (anti-TNF) agent. However, there are not many studies of this type of drug in this disease, although a large study is planned.
Several types of medical procedures may be used to help repair damaged blood vessels and to restore blood flow if major damage from the disease occurs. Angioplasty is a medical procedure in which a balloon is used to open narrowed or blocked blood vessels. A by-pass graft may also be done, which is a surgery performed to redirect a clogged or severely damaged vessel.
Prognosis: TA is a chronic disease. The degree of new disease involvement over time can be quite different between patients, and the inflammation may never completely go away. In some patients the disease eventually ‘burns out' and symptoms and inflammation do not ever return.